Last reviewed: June 2019
Last updated: January  2019

Summary

Definition

History and exam

Key diagnostic factors

  • simultaneous contraction of agonist and antagonist muscles
  • muscle pain
  • appearance or worsening of dystonia with action
  • blepharospasm
  • cervical torticollis
  • hand spasms
  • foot spasms

Other diagnostic factors

  • twisting of the affected body part
  • geste antagoniste (sensory trick)
  • spread to another body part
  • parkinsonism
  • myoclonus
  • tremor, weakness, or spasticity
  • reflex sympathetic dystrophy
  • Kayser-Fleischer rings on slit-lamp examination
  • acute presentation (within 5 days of exposure to antidopaminergic agent)
  • acute worsening of pre-existing generalized dystonia

Risk factors

  • family history of dystonia
  • repetitive activity of affected region
  • birth injury and delayed development in childhood
  • exposure to antidopaminergic agents
  • trauma
  • genetic mutation
  • Ashkenazi Jewish ethnicity
  • structural lesion of the basal ganglia
  • parkinsonian syndrome

Diagnostic investigations

1st investigations to order

  • levodopa responsiveness
Full details

Investigations to consider

  • cranial magnetic resonance imaging
  • serum ceruloplasmin
  • 24-hour urine copper
  • DYT1 gene testing
  • GCH1 gene testing
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Associate Professor of Neurology

Parkinson's Disease and Movement Disorders Center

Beth Israel Deaconess Medical Center

Boston

MA

Disclosures

SF is employed by the Beth Israel Deaconess Medical Center, which has received funding from Allergan to support partial fellowship training.

Dr Samuel Frank would like to gratefully acknowledge Dr Ludy C. Shih, Dr David K. Simon, and Dr Daniel Tarsy, previous contributors to this topic.

Peer reviewers VIEW ALL

Reader in Clinical Neurology

Honorary Consultant

Institute of Neurology

National Hospital for Neurology and Neurosurgery

London

UK

Disclosures

PDL declares that she has no competing interests.

Assistant Professor

Director

Movement Disorder Center

Dept of Neurology

University of Florida

Tampa

FL

Disclosures

ZH has been a consultant for Allergan and a speaker for Novartis and TEVA.

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